4A43.4 Diffuse eosinophilic fasciitis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Also called Shulman disease/diffuse fasciitis, diffuse eosinophilic fasciitis is a rare idiopathic disorder associated with induration of the skin (orange-peel sign) that generally develops rapidly. It is a dermal and hypodermal sclerosis associated with fibrotic thickening of the subcutaneous adipose lobular septa, superficial fascia, and perimysium. Full thickness excisional biopsy of skin lesions revealing fibrosis of the subcutaneous fascia is generally required for diagnosis. Onset follows unusual physical exertion and trauma, especially in males.

synonyms

• Diffuse eosinophilic fasciitis
• Eosinophilic fasciitis
• Fasciitis with eosinophilia syndrome
• Shulman syndrome
• Diffuse fasciitis

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